Vasoactive Intestinal Peptide (VIP), a pulmonary vasodilator and inhibitor of vascular smooth muscle proliferation, is absent in pulmonary arteries of patients with idiopathic pulmonary arterial hypertension (PAH). We previously determined that targeted deletion of the VIP gene in mice leads to PAH with pulmonary vascular remodeling and right ventricular (RV) dilatation. Whether the left ventricle is also affected by VIP gene deletion is unknown. In the current study, we examined if VIP knockout mice (VIP(-/-)) develop both right (RV) and left ventricular (LV) cardiomyopathy, manifested by LV dilatation and systolic dysfunction, as well as overexpression of genes conducive to heart failure.We examined VIP(-/-)and wild type (WT) mice using Magnetic Resonance Imaging (MRI) for evidence of cardiomyopathy associated with biventricular dilation and wall thickness changes. Lung tissue from VIP(-/-) and WT mice was subjected to whole-genome gene microarray analysis.Lungs from VIP(-/-) mice showed overexpression of cardiomyopathy genes: Myh1 was upregulated 224 times over WT, and Mylpf was increased 72 fold. Tnnt3 was increased 105 times and tnnc2 181 fold. Hearts were dilated in VIP(-/-) mice, with thinning of LV wall and increase in RV and LV chamber size, though RV enlargement varied. Weights of VIP(-/-) mice were consistently lower.Critically-important heart failure-related genes are upregulated in VIP(-/-) mice associated with the spontaneous cardiomyopathy phenotype, involving both left and right ventricles, suggesting that loss of the VIP gene orchestrates a panoply of pathogenic genes which are detrimental to both left and right cardiac homeostasis.

History: A 32-year-old woman was admitted to the emergency department because of acute dyspnea and syncope. A few minutes before the onset of symptoms, she had self-administered an intravenous injection of one gram of heroin combined with grinded flunitrazepam tablets.Investigations: Signs of acute cor pulmonale were detected on transthoracic echocardiography despite lack of pulmonary embolism in computed tomography. It was assumed that microembolisms were the cause of acute pulmonary hypertension after intravenous injection of heroin and flunitrazepam.Treatment and course: Because of lack of thrombus in CT scan therapeutic anticoagulation with unfractionated heparin and oxygen insufflation was initiated resulting in rapid improvement of oxygen saturation and blood pressure. On the following day pulmonary pressure in transthoracic echocardiography was already decreased significantly. Without signs of deep venous thrombosis in duplex scan and only a marginal sub segmental perfusion deficit in ventilation-perfusion-scintigraphy therapeutic anticoagulation was recommended for three months.

Conclusion:

The most likely cause of micro embolisms in this case are particles of talc, which are often used to cut heroin, or the microcrystalline cellulose used in tablets. There have been reports of tissue necrosis due to arterial embolism/vasospasm by crystalloid or oily substances (embolia cutis medicamentosa) in the extremities after intraarterial injection of grinded flunitrazepam tablets. Therefore it seems plausible that intravenous application may cause a serve but transient deficit of perfusion in pulmonary circulation.

BACKGROUND:

Emergency surgery itself induces high risk for postoperative mortality and morbidities; however, it remains unknown which concomitant pathological conditions of emergency surgeries are causative factors of deteriorating outcomes. This study examined the causal factors of postoperative mortality and morbidity in cases of emergency surgery.

METHODS:

Patients undergoing emergency surgery from January to December 2007 were enrolled in this retrospective cohort study. Causal relationships were analyzed by stepwise multivariate logistic regression analysis between possible independent factors (sex, age, kind of surgical department, timing of surgery, duration of surgery, blood transfusion, deteriorated consciousness level, shock state, abnormal coagulate state, and history of hypertension, diabetes, ischemic heart disease, chronic obstructive pulmonary disease, renal failure, and anemia) and postoperative mortality or morbidities (failure of removal of tracheal tube after operation, tracheotomy, cerebral infarction, massive hemorrhage, severe hypotension, severe hypoxemia, and severe arrhythmia during or after surgery).

RESULTS:

Shock, deteriorated consciousness level, chronic obstructive lung disease, and ischemic heart disease were significant risk factors for mortality (OR 14.2, 7.9, 6.4, and 3.8, respectively), and deteriorated consciousness level, blood transfusion, shock, chronic obstructive lung disease, diabetes, cardiovascular surgery, and operation longer than 2 h were significant risk factors for morbidity (OR 19.1, 3.3, 3.0, 2.5, 2.4, 2.4, and 1.8, respectively).

CONCLUSION:

State of shock, deteriorated consciousness level, chronic obstructive lung disease, ischemic heart disease, hemorrhage requiring blood transfusion, age over 80 years, cardiovascular surgery, surgeries at night, and surgeries of duration more than 2 h cause patients to be strongly susceptible to postoperative mortality or morbidity in emergency surgeries.

SESSION TYPE: Bronchology Student/Resident Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Thoracic tumors are a common cause of SVC syndrome. The mass can cause complete or partial obstruction of the vessel lumen. The most common malignancies known to cause this syndrome are of bronchogenic origin. Because of the distension and back pressure caused by the tumor occluding the superior vena cava, bleeding is a feared and known complication. There has been no publication that we are aware of looking at EBUS for diagnosis of this condition.

CASE PRESENTATION:

72 year old woman with a past medical history of hypertension presented with cough, facial edema, and dyspnea to our emergency department. Patient had an extensive smoking history, more than 100pkyrs, and quit 6months ago. She also reported anorexia and weight loss. Chest radiography revealed tracheal deviation to the left and mediastinal fullness. Computerized Tomography of the chest was positive for a mediastinal mass invading the proximal part of the superior vena cava ( Fig 1), and the clinical diagnosis of SVC syndrome was made. Patient was treated with steroids. An EBUS guided needle aspiration was performed along the right paratracheal and subcarinal lymph nodes region ( Fig 2). Rapid on site evaluation by a pathologist was diagnostic for non-small cell malignancy, later confirmed as adenocarcinoma of primary pulmonary origin. Radiation therapy was instituted and the patient was discharged to home with scheduled follow-up visits. No complications were noted with the procedure.

DISCUSSION:

We illustrate the safety of EBUS in a case of SVC syndrome. Prior studies were conducted on safety of blind transbronchial needle biopsies in the diagnosis of SVC syndrome with rapid on site evaluation of pathological specimen (Brundyn et al). The use of EBUS while performing the transbronchial biopsy has a dual benefit. The vessels can be identified and avoided during the biopsy procedure increasing safety, and a suitable area of the mass identified increasing yield.

CONCLUSIONS:

EBUS can be performed safely in a patient with SVC syndrome, and has the potential to increase safety and yield compared to other diagnostic procedures.1) Brundyn K, Koegelenberg CF, Diacon AH, et al.Transbronchial fine needle aspiration biopsy and rapid on-site evaluation in the setting of superior vena cava syndrome Diagn Cytopathol. 2011 Nov 18.DISCLOSURE: The following authors have nothing to disclose: Bala Prakash, Shweta Upadhyay, Veronica Brito, Shalinee Chawla, Jonathan IlowiteNo Product/Research Disclosure InformationWinthrop University Hospital, Mineola, NY.

SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Complete unilateral pulmonary agenesis is an extremely rare congenital condition, characterized by complete absence of bronchi, parenchyma, vessels and pleural cavity in the affected lung. Fifty percent of infants born with pulmonary aplasia are stillborn or die within the first five years of life. We present a patient with right pulmonary agenesis complicated by ASD that has survived to adulthood.

CASE PRESENTATION:

A 21 year-old female presents with new onset of hypoxic respiratory failure and dyspnea after diagnosis of community-acquired pneumonia. She has a right pulmonary agenesis, dextrocardia and ASD. Initial diagnosis in the first week of life and ASD surgical repair at 7 months of age, complicated with chronic hypoxic respiratory failure and tracheostomy till age of 5. She also has severe pulmonary hypertension. She was lost to follow up until the current presentation. She was not on supplemental oxygen until current hospitalization. Community-acquired pneumonia treated with Moxifloxacin 10 days course, discharged on 6 L/min of oxygen at rest. Physical exam: BMI 45, vesicular breath sounds over left chest and transmitted breath sounds over right chest, normal S1 and loud S2 over right hemithorax. Echocardiogram: dextrocardia, estimated RV pressure of 89 mmHg with moderate mitral regurgitation. PFT shows obstruction with FEV1/FVC 68%, FEV1 0.81 L (28.7%) and FVC 1.2 L (37.9 %). Chest roenterogram: homogenously opaque right hemithorax with mediastinal shift and hyperlucency of left lung field. CT chest: left lung emphysema, right mediastinal shift, right lung agenesis and heart in right hemithorax.

DISCUSSION:

There is no specific therapy for pulmonary agenesis. We took a symptomatic approach to therapy, treating her obstructive lung disease with Albuterol, Tiotropium, and Mometasone, pulmonary rehabilitation with improved functional status. Planned left and right cardiac catheterization to evaluate for any remediable anatomic cardiovascular problems that may not be evident by ECHO but may contribute to pulmonary hypertension and assess responsiveness to pulmonary vasodilators.

CONCLUSIONS:

Lung aplasia is often associated with acute respiratory distress and a high mortality rate early in life. This case highlights the fact that children with complicated congenital pulmonary diseases are increasingly living into adulthood. As adult pulmonologists, we must be more aware of these diseases and their pathophysiological changes to be able to adequately diagnose and treat our patients.1) Skandalakis JE, Gray SW, Symbas P: The trachea and the lungs. In: Skandalakis JE, Gray SW, editors. Embryology for Surgeons. 2nd ed. Baltimore, MD: Williams and Wilkins; 1994. p 429-32.2) Krivchenya DU, Rudenko EO, Lysak SV, Dubrovin AG, Khursin VN, Krivchenya TD. Lung aplasia: Anatomy, history, diagnosis and surgical management. Eur J Pediatr Surg 2007;17:244-50.3) Kumar B, Kandpal DK, Sharma C, Sinha DD. Right lung agenesis. Afr J Paediatr Surg 2008;5:102-4DISCLOSURE: The following authors have nothing to disclose: Olena Lineberry, P.Jim Murphy, Craig Piquette, Kristina BaileyNo Product/Research Disclosure InformationUNMC, Omaha, NE.

SESSION TYPE: DVT/PE/Pulmonary Hypertension Posters IIPRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM

PURPOSE:

To demonstrate that pleural effusion in the setting of pre-capillary pulmonary hypertension does not occur without evidence of pulmonary venous hypertension or development of ascites with transdiaphragmatic migration of fluid to the pleural space.

METHODS:

Bedside ultrasonography was performed on patients immediately prior to undergoing right heart catheterization to look for fluid in three compartments: pleural, pericardial, and peritoneal spaces. Right heart catheterization measurements and diagnoses were subsequently obtained for analysis. Statistical analysis of data was performed using SigmaStat 11.0 or SAS software.

RESULTS:

A total of 22 patients were enrolled. Of those, 17 were female and 5 were male, 11 were African American and 11 were Caucasian. Of the 22 patients, 18 met the criteria for pulmonary hypertension with mean PAP greater than 25mmHg. Of those 18 patients, 14 met criteria for pre-capillary pulmonary hypertension with PCWP less than 15mmHg. The mean PAP was 40.8mmHg. The mean PCWP was 12.5mmHg. Nine patients had small pericardial effusions, and none had moderate or large pericardial effusions. Four patients had pleural effusions, with 3 being small and 1 being small-moderate. Of those 4 patients, 3 had PCWP greater than 15mmHg. No patients demonstrated ascites. Diagnoses for pulmonary hypertension listed include: 6 idiopathic, 6 scleroderma, 3 idiopathic pulmonary fibrosis, 3 pulmonary venous hypertension, 2 portal vein thrombosis, 1 mixed connective tissue disease, 1 sickle cell disease.

CONCLUSIONS:

In our 14 patients with pre-capillary pulmonary hypertension as described by elevated mean PAP and PCWP less than 15mmHg, 6 patients had small pericardial effusions and 1 had right sided pleural effusion. The patient with the pleural effusion was also on a tyrosine kinase inhibitor which can cause isolated pleural effusion. Overall, our hypothesis has held true that patients with pre-capillary hypertension should not develop pleural effusions without another insult.

CLINICAL IMPLICATIONS:

Understanding the true pathogenesis of pleural and pericardial effusion formation in patients with pre-capillary pulmonary hypertension is essential to efficient and effective management.DISCLOSURE: The following authors have nothing to disclose: Edward Kilb, John Huggins, Kristin Highland, Matthew Divietro, Steven SahnNo Product/Research Disclosure InformationMUSC, Charleston, SC.

SESSION TYPE: COPD: Diagnosis and EvaluationPRESENTED ON: Sunday, October 21, 2012 at 01:15 PM - 02:45 PM

PURPOSE:

Heart rate recovery (HRR) after exercise indicates vagal reactivation and has been shown to predict adverse outcomes in patients with idiopathic pulmonary fibrosis and pulmonary arterial hypertension. Our objectives were to determine values of HRR after six-minute walk test (6MWT) in patients with COPD and to determine its association with pulmonary limitation and exercise performance.

METHODS:

HRR1 and HRR2 were defined as the difference in heart rate at the end of a 6MWT and at 1- and 2-minutes of rest respectively. From 2008 to 2011, 80 consecutive patients with COPD underwent 6MWT and were included in the analysis. Logistic regression was used to identify prognostic value of abnormal HRR, chronotropic response, and clinical worsening.

RESULTS:

The mean age of this patient group was 65.4 years. Compared with patients in GOLD stage 3 and 4 (n=59), those with GOLD stage 1 and 2 COPD (n=21) had significantly better 6MW distance (1231.05 meters VS 913.07 meters VS, p < 0.0001). There was no statistically significant difference in heart rate at the end of 6MWT (107 VS 106, p = 0.44). Compared to patients with HRR1 ≥ 13 (n=38), those with HRR 1 < 13 (n=42) had worse FEV1% (38.7% VS 48.3%, p = 0.011) and lower 6MW distance (878.9 meter VS 1126.5 meters, p = 0.0006).

CONCLUSIONS:

Reduced HRR1 after 6MWT is associated with lower % predicted FEV1 and reduced 6MW distance in patients with COPD. Further study is needed to determine prognostic value of HRR1 in patients with COPD.

CLINICAL IMPLICATIONS:

HRR1 has good correlation with % predicted FEV1 and 6MW distance in COPD patients.DISCLOSURE: The following authors have nothing to disclose: Danai Khemasuwan, Kevin McCarthy, Omar MinaiNo Product/Research Disclosure InformationCleveland Clinic Foundation, Cleveland, OH.

SESSION TYPE: DVT/PE/Pulmonary Hypertension Posters IIPRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM

PURPOSE:

Computed tomography of the chest for pulmonary embolism (CTPE) is frequently used in emergency departments (ED) for patients with respiratory complaints. We theorized that the combination of formal screening scores, such as the Pulmonary Embolism Rule-out Criteria (PERC), the revised Geneva score (RGS), coupled with chest radiographs (CXR) would optimize PE diagnosis in the ED.

METHODS:

We performed a retrospective cohort study of consecutive ED patients who underwent CTPE (January - June 2010). We retrospectively scored PERC and RGS. RGS was considered low probability (LP) if <4. CXRs were evaluated and findings were dichotomized into those representing potential alternate diagnoses (pulmonary edema, consolidation, pleural effusion) and all others. We compared the ability of scoring tools alone (PERC and RGS) and then in combination with CXR findings (PERC+CXR, RGS+CXR) to exclude PE. Area under the receiver operating characteristic curves (AUROCs) and negative predictive values (NPVs) served as endpoints.

RESULTS:

The cohort included 776 subjects (mean age: 50.6 + 16.5 years; female: 71.6%; 6.6% diagnosed with PE) and 58.8% (n=456) had a concurrent CXR. In the entire cohort, PERC was LP in 22.9% and RGS was LP in 41.0%. Among non-LP PERC patients, 7.9% had a PE compared to 8.7% of non-LP patients by RGS (p=0.61). NPVs were similar (97.8% for PERC, 96.5% for RGS). The rate of PE in LP RGS patients was 3.5% vs 2.2% for PERC (p=0.45). When CXR demonstrated an alternative diagnosis, 5.5% were still diagnosed with PE compared to 5.7% without alternative diagnoses (p=0.93). When either RGS or PERC was LP and CXR showed an alternate diagnosis, no subject had a PE. The AUROCs for PERC and RGS were 0.71 vs 0.63, respectively. When CXR was added to PERC and RGS, AUROCs were similar.

CONCLUSIONS:

RGS and PERC have similar diagnostic yield for PE. When combined with radiographic findings for alternative diagnoses, both PERC and RGS can safely rule out PE.

CLINICAL IMPLICATIONS:

When PERC or RGS are low probability with a concomitant CXR demonstrating an alternative diagnosis, the likelihood for PE is low. Such screening tools can be used to exclude PE and minimize use of CTPE in the ED.DISCLOSURE: The following authors have nothing to disclose: Genese Lamare, A. Shorr, Chee ChanNo Product/Research Disclosure InformationWashington Hospital Center, Washington, DC.

SESSION TYPE: Cancer Student/Resident Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

While fludeoxyglucose-avid lung nodules often represent cancer, a broader differential diagnosis must be considered.

CASE PRESENTATION:

A 74 year old woman presented with multiple, progressive symptoms of lower motor neuron leg weakness, left ptosis, papular rash on the extremities, weight loss and a fludeoxyglucose-avid lung nodule. Past medical history was significant for malignant melanoma 11 years prior, colon cancer 8 years prior, and hypertension. She was a lifetime non-smoker. Her only medication was metoprolol. On physical examination, vital signs, cardiac and pulmonary systems were unremarkable. There was no lymphadenopathy or hepatosplenomegaly. Neurologic examination revealed a left ptosis and 4+/5 weakness and decreased sensation to light touch in the bilateral lower extremities with a distal, symmetric distribution. Multiple non-tender erythematous papules over the arms and legs were noted. Complete blood count, serum creatinine and urinalysis were unremarkable. Carcinoembryonic antigen level, anti-nuclear antibody, anti-neutrophil cytoplasmic antibodies (ANCA), erythrocyte sedimentation rate and C-reactive protein were normal. Computed tomography of the chest showed a spiculated 1.6 cm right lower lobe pulmonary nodule, and innumerable diffuse bilateral pulmonary nodules measuring up to 6 mm. Only the dominant nodule was found to be hypermetabolic on a subsequent positron emission tomography scan (standardized uptake value 8.6). A transthoracic needle aspiration of the lung nodule demonstrated a lymphohistiocytic infiltrate with poorly formed granulomas and necrosis. Grocott's methenamine silver and acid-fast bacillus stains were negative. No malignant cells were seen. Flow cytometry demonstrated no clonal proliferation, and Epstein-Barr virus (EBV) stains were negative. Multiple skin biopsies demonstrated an atypical lymphohistiocytic infiltrate with granulomatous features. A repeat biopsy of the same lung nodule revealed identical pathology. A diagnosis of probable ANCA-negative systemic vasculitis was made, with pulmonary, neurologic and cutaneous manifestations. The patient was initiated on corticosteroid therapy with significant improvement in her symptoms.

DISCUSSION:

Lymphoma and related lymphoid granulomatosis are less likely given normal flow cytometry and the lack of EBV, respectively. Conversely, the lack of positive serum markers does not rule out vasculitis. Routine immunsuppression by corticosteroids may be inadequate in the treatment of lymphohistiocytic lung tumor. Aggressive efforts may be required to break the cycle of lymphocyte-histiocyte activation, potentially by cell-specific monoclonal antibodies.

CONCLUSIONS:

Inflammatory lung tumors, although rare, may mimic malignant tumors both clinically and radiologically. Clinical suspicion and tissue biopsies are required for the diagnosis.1) Brown KK. Pulmonary Vasculitis. Proc Am Thoracic Soc. 3(1): 48-57. 2006DISCLOSURE: The following authors have nothing to disclose: Ryan Van Wert, Daya UpadhyayNo Product/Research Disclosure InformationStanford University, Stanford, CA.

SESSION TYPE: DVT/PE/Pulmonary Hypertension Posters IIPRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM

PURPOSE:

Pulmonary arterial hypertension (PAH) is a rare disease with high morbidity and mortality. There are multiple associated conditions, each with unique risk factors for development of the disease. Previous studies have suggested that insulin resistance is highly prevalent in patients with PAH; the long-term impact of this finding on survival in PAH is unclear. We aimed to investigate differences in mortality between PAH patients with and without diabetes.

METHODS:

From a prospective registry of PAH patients seen at our institution, we analyzed the effect of diabetes mellitus (DM) on survival. DM was defined as any of the following: hemoglobin A1c of 6.0% or greater, patients who had a random non-fasting glucose value of greater than 200mg/dL, or patients who were treated with an FDA-approved medication for DM. Patients who met none of these criteria were defined as non-diabetic (NDM). PAH was diagnosed according to standard criteria and only patients with idiopathic (IPAH) or hereditary (HPAH) PAH and right heart catheterization (RHC) data were included in this analysis.

RESULTS:

A total of 113 patients were analyzed: 29 with DM and 84 NDM. Baseline characteristics were similar between diabetic and non-diabetic PAH patients. Gender, age at diagnosis of PAH, PAH type and duration, and 6 minute walk distance were similar between groups. BMI was similar in the two groups (31.4 ± 6.78 vs 29.8 ± 8.3 kg/m2, p=0.34), as was BNP (382 ± 630 vs 282 ± 434 pg/mL). RHC data did not differ significantly between the two groups. However, a significant survival difference at 10 years was found between the two groups (p=0.04 by log rank).

CONCLUSIONS:

Utilizing data from a large database of PAH patients, we show reduced survival in IPAH and HPAH patients with DM compared to those without.

CLINICAL IMPLICATIONS:

Further work should be directed at determining whether available antidiabetic medications may impact the course of PAH; work to elucidate a pathologic mechanism of this mortality difference would also be recommended.DISCLOSURE: The following authors have nothing to disclose: Levi Benson, Meredith Pugh, Eric Austin, Kelly Fox, Lisa Wheeler, Evan Brittain, Ivan Robbins, Anna HemnesNo Product/Research Disclosure InformationVanderbilt University, Nashville, TN.